OBSOLETE: Congenital myopathy with fiber size variation

Last reviewed

🖨 Print for my doctorAdvocacy Hub →
ORPHA:172982
Who is this for?
Show terms as
8Treatment centers

Where are you in your journey?

UniteRare data is sourced from FDA.gov, ClinicalTrials.gov, Orphanet, OMIM, and NORD.
Report missing data

Overview

Congenital myopathy with fiber size variation is a rare inherited muscle disorder that is present from birth or early infancy. The term 'OBSOLETE' in its name means that this condition has been reclassified in medical databases and may now fall under a broader category of congenital myopathies. Congenital myopathies are a group of muscle diseases caused by problems in the structure or function of muscle fibers. In this particular condition, a muscle biopsy shows that the muscle fibers vary significantly in size, which is not normal. This variation in fiber size leads to muscle weakness, typically affecting the limbs, face, and sometimes the muscles used for breathing. Patients with this condition usually show signs of muscle weakness early in life, such as low muscle tone (floppiness) at birth, difficulty feeding, delayed motor milestones like sitting or walking, and general muscle weakness. The severity can range from mild to severe. Some children may have difficulty breathing on their own and may need respiratory support. There is currently no cure for congenital myopathies, including this subtype. Treatment focuses on managing symptoms and improving quality of life. This includes physical therapy to maintain muscle strength and flexibility, respiratory support if breathing muscles are affected, and nutritional support if feeding is difficult. Because this diagnosis has been reclassified, patients previously diagnosed with this condition should work with their medical team to determine if a more specific genetic diagnosis is now available, which could guide more targeted management.

Key symptoms:

Muscle weakness from birthLow muscle tone (floppiness)Delayed motor milestones like sitting and walkingDifficulty feeding as a babyBreathing difficultiesFacial muscle weaknessThin or underdeveloped musclesJoint contractures (stiff joints)Scoliosis (curved spine)Fatigue and low enduranceDifficulty swallowingHigh-arched palate

Inheritance

Variable

Can be inherited in different ways depending on the underlying gene

Age of Onset

Neonatal

Begins at or shortly after birth (first 4 weeks)

Orphanet ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for OBSOLETE: Congenital myopathy with fiber size variation.

View clinical trials →

No actively recruiting trials found for OBSOLETE: Congenital myopathy with fiber size variation at this time.

New trials open frequently. Follow this disease to get notified.

Search ClinicalTrials.gov ↗Join the OBSOLETE: Congenital myopathy with fiber size variation community →

No specialists are currently listed for OBSOLETE: Congenital myopathy with fiber size variation.

View NORD Rare Disease Centers ↗Undiagnosed Disease Network ↗

Treatment Centers

8 centers
🏥 NORD

Baylor College of Medicine Rare Disease Center

Baylor College of Medicine

📍 Houston, TX

🏥 NORD

Stanford Medicine Rare Disease Center

Stanford Medicine

📍 Stanford, CA

🔬 UDN

NIH Clinical Center Undiagnosed Diseases Program

National Institutes of Health

📍 Bethesda, MD

🔬 UDN

UCLA UDN Clinical Site

UCLA Health

📍 Los Angeles, CA

🔬 UDN

Baylor College of Medicine UDN Clinical Site

Baylor College of Medicine

📍 Houston, TX

🔬 UDN

Harvard/MGH UDN Clinical Site

Massachusetts General Hospital

📍 Boston, MA

🏥 NORD

Mayo Clinic Center for Individualized Medicine

Mayo Clinic

📍 Rochester, MN

👤 Mayo Clinic Center for Individualized Medicine

🏥 NORD

UCLA Rare Disease Day Program

UCLA Health

📍 Los Angeles, CA

Travel Grants

No travel grants are currently matched to OBSOLETE: Congenital myopathy with fiber size variation.

Search all travel grants →NORD Financial Assistance ↗

Community

Open OBSOLETE: Congenital myopathy with fiber size variationForum →

No community posts yet. Be the first to share your experience with OBSOLETE: Congenital myopathy with fiber size variation.

Start the conversation →

Latest news about OBSOLETE: Congenital myopathy with fiber size variation

No recent news articles for OBSOLETE: Congenital myopathy with fiber size variation.

Follow this condition to be notified when news becomes available.

Caregiver Resources

NORD Caregiver Resources

Support, advocacy, and financial assistance for caregivers of rare disease patients.

Mental Health Support

Rare disease caregiving can be isolating. Connect with counseling and peer support.

Family & Caregiver Grants

Financial assistance programs specifically for caregivers of rare disease patients.

Social Security Disability

Learn how rare disease patients may qualify for SSDI/SSI benefits.

Questions for your doctor

Bring these to your next appointment

  • Q1.Has genetic testing been done to identify the specific gene causing this condition?,Since this diagnosis is now considered obsolete, is there a more specific diagnosis that applies to my child?,How often should my child's breathing and lung function be monitored?,What physical therapy program is recommended, and how often should sessions occur?,Are there any clinical trials or new treatments being studied for this type of myopathy?,What emergency signs should I watch for, and what should I do if they occur?,Should other family members be tested for this condition?

Common questions about OBSOLETE: Congenital myopathy with fiber size variation

What is OBSOLETE: Congenital myopathy with fiber size variation?

Congenital myopathy with fiber size variation is a rare inherited muscle disorder that is present from birth or early infancy. The term 'OBSOLETE' in its name means that this condition has been reclassified in medical databases and may now fall under a broader category of congenital myopathies. Congenital myopathies are a group of muscle diseases caused by problems in the structure or function of muscle fibers. In this particular condition, a muscle biopsy shows that the muscle fibers vary significantly in size, which is not normal. This variation in fiber size leads to muscle weakness, typica

At what age does OBSOLETE: Congenital myopathy with fiber size variation typically begin?

Typical onset of OBSOLETE: Congenital myopathy with fiber size variation is neonatal. Age of onset can vary across affected individuals.